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[原发性弥漫性脑膜黑色素瘤病。病例报告]

[Primary diffuse meningeal melanomatosis. Case report].

作者信息

Bajer-Czajkowska Anna, Nowacki Przemysław

机构信息

Katedra i Klinika Neurologii, Pomorska Akademia Medyczna w Szczecinie, Szczecin.

出版信息

Neurol Neurochir Pol. 2007 Jan-Feb;41(1):82-8.

Abstract

Melanoma malignum constitutes only 0.1% of central nervous system neoplasms. It can occur either as a solid tumour or as a diffuse meningeal melanomatosis. A case of the latter form of central nervous system melanoma is presented in a 44-year-old man, suffering from headaches, cerebrospinal fluid protein elevation, optic disc oedema, hydrocephalus, seizures, cranial nerves and multilevel spinal root damage. Above mentioned neurological manifestations gradually increased within 18 months after onset of first symptoms of the disease (headache). The clinical course in our patient suggested diffuse leptomeningeal involvement. Despite the use of detailed diagnostic procedures, the correct diagnosis of primary diffuse meningeal melanomatosis was established at postmortem examination. We present our case because of the casuistic rarity of primary diffuse meningeal melanomatosis coexisting with obstructive hydrocephalus resulting from, among other things, extensive neoplastic infiltration of the vertebral channel, especially the cauda equina.

摘要

恶性黑色素瘤仅占中枢神经系统肿瘤的0.1%。它可以表现为实体瘤,也可以表现为弥漫性脑膜黑色素沉着症。本文介绍了一名44岁男性患者,患有中枢神经系统黑色素瘤的后一种形式,表现为头痛、脑脊液蛋白升高、视盘水肿、脑积水、癫痫发作、颅神经和多节段脊神经根损伤。上述神经系统表现于疾病首发症状(头痛)出现后的18个月内逐渐加重。我们患者的临床病程提示弥漫性软脑膜受累。尽管采用了详细的诊断程序,但原发性弥漫性脑膜黑色素沉着症的正确诊断是在尸检时确定的。我们之所以介绍这个病例,是因为原发性弥漫性脑膜黑色素沉着症与梗阻性脑积水并存的情况非常罕见,梗阻性脑积水是由椎管尤其是马尾的广泛肿瘤浸润等原因引起的。

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